The authors present a case of primary uterine reticulum cell sarcoma who developed disseminated disease 6 years after undergoing a total abdominal hysterectomy. Review of the literature reveals 27 other patients with uterine lymphomas. Abnormal vaginal bleeding was the presenting complaint in 66% (14/21) of patients. Reticulum cell sarcoma was the most frequent histologic type, 68% (19/28). 28% of patients were alive and well 6–24 years after therapy.